Interruption of the aortic arch is a rare and usually lethal cardiac malformation. We report a rare case of a patient with IAA distal to the left subclavian artery associated with double outlet right ventricle, subaortic VSD and patent DA who survived to adulthood. In this patient, the complete diagnosis was made by cardiac catheterization and angiocardiography. We discuss the probable mechanisms, diagnostic problems and therapeutic implications of such long survival.